{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T16:34:57Z","timestamp":1793118897734,"version":"4.5.0"},"reference-count":64,"publisher":"Bioscientifica","issue":"10","license":[{"start":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T00:00:00Z","timestamp":1791936000000},"content-version":"vor","delay-in-days":13,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Natural Science Foundation of China","award":["82570929, 82170806"],"award-info":[{"award-number":["82570929, 82170806"]}],"id":[{"id":"https:\/\/ror.org\/01h0zpd94","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Natural Science Foundation of Hunan Province","award":["2023JJ30915, 2025JJ20089"],"award-info":[{"award-number":["2023JJ30915, 2025JJ20089"]}]}],"content-domain":{"domain":["journals.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2026,10,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Therapeutic options for metastatic pheochromocytomas and paragangliomas (mPPGLs) remain limited. Recent studies indicate that tyrosine kinase inhibitors (TKIs) offer a promising treatment approach. This study aims to evaluate the efficacy and safety of TKIs for mPPGLs based on available evidence. A systematic search for studies investigating the treatment of mPPGLs with TKIs was conducted across PubMed, Embase, and Web of Science from database inception to February 2026. Data were synthesized using fixed- or random-effects meta-analysis model. Subgroup analysis was conducted based on the status of the SDHB gene and subtypes of TKIs. A total of 11 studies, comprising 4 observational and 7 interventional studies, with 246 patients involved. The stable disease rate, objective response rate (ORR), and disease control rate (DCR) were 51.8% (95% CI: 44.6\u201359.1%), 27.7% (95% CI: 21.6\u201334.3%), and 84.2% (95% CI: 74.8\u201391.9%), respectively. The proportion of discontinuation due to adverse events was 11.3% (95% CI: 5.4\u201318.7%). The combined median progression-free survival and overall survival were 13.4 (95% CI: 7.9\u201314.7) and 26.7 (95% CI: 24.9\u201349.4) months. Pooled ORR (36.8% (95% CI: 21.1\u201353.8%) vs 20.1% (95% CI: 10.8\u201331.0%), P = 0.06) and DCR (92.2% (95% CI: 79.6\u201399.7%) vs 78.5% (95% CI: 57.3\u201394.5%); P = 0.269) were higher in patients with SDHB-related mPPGL than in those with non-SDHB mPPGL, although the differences were not statistically significant. Notably, sunitinib showed no therapeutic superiority over other TKI subtypes, with lower pooled ORR (21.2% (95% CI: 14.0\u201329.4%) vs 36.9% (95% CI: 26.9\u201347.4%); P = 0.04) and DCR (72.6% (95% CI: 63.9\u201380.6%) vs 94.5% (95% CI: 87.2\u201399.3%); P = 0.001). Consequently, TKIs show promising efficacy and manageable adverse event rates in mPPGL, supporting TKIs as components of the systematic treatment.<\/jats:p>","DOI":"10.1530\/erc-26-0201","type":"journal-article","created":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T14:58:46Z","timestamp":1791989926000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-8","source":"Crossref","is-referenced-by-count":0,"title":["Tyrosine kinase inhibitors in metastatic pheochromocytomas and paragangliomas: an updated systematic review and meta-analysis"],"prefix":"10.1530","volume":"33","author":[{"given":"Xiaowen","family":"Xu","sequence":"first","affiliation":[{"name":"Department of Urology, Xiangya Hospital, Central South University 1 , ,","place":["Changsha, China"]},{"name":"National Clinical Research Center for Geriatric Disorders (Xiangya Hospital), Central South University 2 , ,","place":["Changsha, 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China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Herve Pasteur","family":"Ndikuriyo","sequence":"additional","affiliation":[{"name":"Department of Urology, Xiangya Hospital, Central South University 1 , ,","place":["Changsha, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yizhou","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Urology, Xiangya Hospital, Central South University 1 , ,","place":["Changsha, China"]},{"name":"National Clinical Research Center for Geriatric Disorders (Xiangya Hospital), Central South University 2 , ,","place":["Changsha, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kai","family":"Cheng","sequence":"additional","affiliation":[{"name":"Department of Urology, Xiangya Hospital, Central South University 1 , ,","place":["Changsha, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhi","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Urology, Xiangya Hospital, Central South University 1 , ,","place":["Changsha, China"]},{"name":"National Clinical Research Center for Geriatric Disorders (Xiangya Hospital), Central South University 2 , ,","place":["Changsha, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junjie","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Urology, Xiangya Hospital, Central South University 1 , ,","place":["Changsha, China"]},{"name":"National Clinical Research Center for Geriatric Disorders (Xiangya Hospital), Central South University 2 , ,","place":["Changsha, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minghao","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Urology, Xiangya Hospital, Central South University 1 , ,","place":["Changsha, China"]},{"name":"National Clinical Research Center for Geriatric Disorders (Xiangya Hospital), Central South University 2 , ,","place":["Changsha, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiao","family":"Xiao","sequence":"additional","affiliation":[{"name":"Department of Urology, Jiangxi Provincial People\u2019s Hospital 3 , ,","place":["Nanchang, China"]},{"name":"Department of Physiology, College of Medicine and Health, University College Cork 4 , ,","place":["Cork, Ireland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0274-4057","authenticated-orcid":true,"given":"Longfei","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Urology, Xiangya Hospital, Central South University 1 , ,","place":["Changsha, China"]},{"name":"National Clinical Research Center for Geriatric Disorders (Xiangya Hospital), Central South University 2 , ,","place":["Changsha, 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